The first clinical application of the spiral intestinal lengthening and tailoring (silt) in extreme short bowel syndrome.

AIM: Spiral Intestinal Lengthening and Tailoring (SILT) invented by our team is a new technique that offers minimal mesenteric handling and a more physiological result compared to the STEP procedure. Its feasibility has been tested in animal models and now we report the first successful human application in extreme short bowel syndrome. MATERIALS AND METHODS: A 3-year-old girl suffered subtotal loss of her small bowel and ileocaecal junction as a result of midgut volvulus. Only 15 cm of jejunum remained intact. Parenteral nutrition (PN), gastrostomy feeding, controlled bowel expansion and SILT procedure were applied. RESULTS: The length of the jejunum increased from the initial 15 to 22 cm during 12 months of PN and bowel expansion. Eleven centimeter of distended bowel was further lengthened up to 20 cm by SILT giving a total small bowel length of 31 cm. Oral and gastrostomy feedings were commenced 5 days postoperatively. There were no surgical complications 6 months after the procedure. The patient's liver function was preserved, she was weaned off PN, discharged from hospital, but remained on gastrostomy top up feeding. The net weight gain of the patient was 1,800 g 6 months after the procedure. CONCLUSION: SILT procedure is a safe and feasible technique for human intestinal lengthening and tailoring.

National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary--clinical characteristics and disease course of 44 patients with juvenile dermatomyositis.

Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs. Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM). A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Although we found favourable survival probability, further investigations are needed to assess functional outcome.

Glycosaminoglycan excretion in connective tissue diseases.

Abnormal glycosaminoglycan metabolism has been widely studied in cases of mucopolysaccharidoses (MPSD) with increased acid glycosaminoglycan (aGAG) excretion. A disorder in aGAG metabolism can be confirmed in several diseases with known etiology. We have carried out a comparative study on the urinary aGAG output in systemic connective tissue diseases and in childhood cases of MPSD. In children suffering from rheumatoid arthritis or scleroderma, the urinary aGAG output did not surpass 80% of the value excreted in MPSD; furthermore, 20 to 30% was uronic acid. In the case of osteogenesis imperfecta the remarkably high amount of excreted aGAG contained over 60% uronic acid.